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Titolo:
DESMIN IN MUSCLE FORMATION AND MAINTENANCE - KNOCKOUTS AND CONSEQUENCES
Autore:
CAPETANAKI Y; MILNER DJ; WEITZER G;
Indirizzi:
BAYLOR COLL MED,DEPT CELL BIOL,1 BAYLOR PLAZA HOUSTON TX 77030
Titolo Testata:
Cell structure and function
fascicolo: 1, volume: 22, anno: 1997,
pagine: 103 - 116
SICI:
0386-7196(1997)22:1<103:DIMFAM>2.0.ZU;2-R
Fonte:
ISI
Lingua:
ENG
Soggetto:
INTERMEDIATE FILAMENT PROTEIN; EPIDERMOLYSIS-BULLOSA SIMPLEX; CHICKEN SKELETAL-MUSCLE; AMYOTROPHIC-LATERAL-SCLEROSIS; MATRIX ATTACHMENT REGIONS; GENE-EXPRESSION; TRANSGENIC MICE; PLASMA-MEMBRANE; NUCLEAR-ENVELOPE; SMOOTH-MUSCLE;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
125
Recensione:
Indirizzi per estratti:
Citazione:
Y. Capetanaki et al., "DESMIN IN MUSCLE FORMATION AND MAINTENANCE - KNOCKOUTS AND CONSEQUENCES", Cell structure and function, 22(1), 1997, pp. 103-116

Abstract

Desmin, the muscle-specific member of the intermediate filament (IF) family, is one of the earliest known myogenic markers in both skeletalmuscle and heart. Its expression precedes that of all known muscle proteins including the members of the MyoD family of myogenic helix-loop-helix (mHLH) regulators with the exception of myf5. In mature striated muscle, desmin Ifs surround the Z-discs, interlink them together andintegrate the contractile apparatus with the sarcolemma and the nucleus. In vitro studies using both antisense RNA and homologous recombination techniques in embryonic stem (ES) cells demonstrated that desmin plays a crucial role during myogenesis, as inhibition of desmin expression blocked myoblast fusion and myotube formation. Both in C2C12 cells and differentiating embryoid bodies, the absence of desmin interferes with the normal myogenic program, as manifested by the inhibition ofthe mHLH transcription regulators, To investigate the function of desmin in all muscle types in vivo, we generated desmin null mice throughhomologous recombination. Surprisingly, a considerable number of these mice are viable and fertile, potentially due to compensation by vimentin, nestin or synemin. However, desmin null mice demonstrate a multisystem disorder involving cardiac, skeletal and smooth muscle, beginning early in their postnatal life. Histological and electron microscopic analysis in both heart and skeletal muscle tissues reveals severe disruption of muscle architecture and degeneration. Structural abnormalities include loss of lateral alignment of myofibrils, perturbation of myofibril anchorage to the sarcolemma, abnormal mitochondrial number and organization, and loss of nuclear shape and positioning. Loose celladhesion and increased intercellular space are prominent defects. Theconsequences of these abnormalities are most severe in the heart, which exhibits progressive degeneration and necrosis of the myocardium accompanied by extensive calcification. Abnormalities of smooth muscle included hypoplasia and degeneration. There is a direct correlation between severity of damage and muscle usage, possibly due to increased susceptibility to normal mechanical damage and/or to repair deficiency in the absence of desmin. In conclusion, the studies so far have demonstrated that though desmin is absolutely necessary for muscle differentiation in vitro, muscle development can take place in vivo in the absence of this intermediate filament protein. However, desmin seems to play an essential role in the maintenance of myofibril, myofiber and whole muscle tissue structural and functional integrity.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 22/09/20 alle ore 20:17:21